Introduction: Pediatric pulmonary arterial hypertension (PAH) can result from a complex array of etiologies, from neonatal persistent PAH to congenital heart disease (CHD), genetic mutations, and acquired forms from lung disease or thrombosis. Such patients may decompensate quickly and require ECMO. We aimed to review a pediatric PAH population requiring ECMO at a single center. Methods: We performed a retrospective study of pediatric patients aged 0-18 years with PAH requiring ECMO from January 1, 2020 through May 15, 2025. PAH therapies initiated prior to, during and after ECMO, and epidemiology and mortality of PAH patients receiving ECMO were reviewed. Results: Thirty-one unique PAH patients required 34 ECMO runs: 29 VA, 2 VV, 2 ECPR; 3 transitioned to oxy-right ventricular assist devices (RVAD). Survival to decannulation was 84%, with 64.5% surviving to discharge. Mean duration of oxygenator use (ECMO and RVAD) was 25.7 days (range: 4-143 days). There were 17 neonates, with 82% survival to decannulation. Two pulmonary embolism patients required ECPR, both surviving to discharge. Three patients with single ventricle cardiac disease (2 Glenn and 1 Fontan) with PAH had 25% survival to decannulation. Two patients had delayed diagnosis of CHD with large atrial shunts and suprasystemic PAH, one of whom was unable to separate from cardiopulmonary bypass (CPB) following repair and the other who required ECMO due to pneumonia but was decannulated at time of shunt repair on CPB. Both survived to discharge. 100% (31/31) of patients received at least one PAH therapy prior to and during ECMO [31/31 (100%) inhaled nitric oxide or inhaled epoprostenol; 26/31 (84%) sildenafil, tadalafil or riociugat; 18/31 (58%) macitentan, ambrisentan or bosentan; 8/31(26%) selexipag; 10/31(32%) treprostinil. 88% (23/26) received at least one PAH therapy after decannulation. Conclusions: Pediatric PAH is a complex disease due to varied etiologies often requiring multiple medications to effectively manage. Certain subpopulations such as those with single ventricle CHD have higher mortality especially when requiring ECMO. Further study is necessary to determine if earlier identification and management of PAH patients prior to ECMO can improve survival and decrease need for mechanical circulatory support in this high-risk population.
Miles et al. (Sun,) studied this question.